Laryngeal atresia is a generally fatal congenital anomaly with an incidence of 1:50000 per birth. This congenital anomaly is a condition of multifactorial inheritance, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphragma, fetal hydrops and ascites. The diagnosis is usually made when there is failure to perform endotracheal intubation in a neonate with severe respiratory distress and absence of audible cry. Here, we present a very rare case of a newborn with laryngeal atresia having respiratory distress and was sustained the first few minutes of life by partial ventilation via persistent pharyngotracheal duct. We would like to draw the attention of all clinicians on this issue we are offering a rare fatal cases of newborns with congenital presentation.